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Patients diagnosed with essential thrombocythemia (ET) or polycythemia vera (PV) are at risk of progression to secondary myelofibrosis (MF).1 Depending on initial diagnosis, subsequent disease is either classified as post-ET MF or post-PV MF; however, clinical features remain similar to those of primary MF.1 Currently, there are very few prospective studies investigating patients with either post-ET or post-PV MF, and a prognostic model for primary MF may aid in the differentiation of secondary disease.1
Shide et al.1 performed a nationwide, longitudinal, prospective survey in Japan to clarify clinical characteristics associated with patients diagnosed with post-ET and post-PV MF. Here, we summarize the key results.
Table 1. Clinical characteristics that differ significantly between patients with post-ET and post-PV MF*
Characteristic, % (unless otherwise stated) |
Post-ET MF |
Post-PV MF |
p value |
---|---|---|---|
Splenomegaly |
68.9 |
81.2 |
0.024 |
Median WBC, ×109/L |
8,820 |
14,400 |
<0.001 |
WBC >25, ×109/L |
11.7 |
23.1 |
0.012 |
Median Hb, g/L |
9.4 |
10.7 |
<0.001 |
Hb <10, g/dL |
60.4 |
41.9 |
0.002 |
Median platelets, ×109/L |
38.3 |
27.4 |
<0.001 |
JAK2V617F mutation |
56.3 |
96.2 |
<0.001 |
ET, essential thrombocythemia; Hb, hemoglobin; JAK2, Janus kinase 2; MF, myelofibrosis; PV, polycythemia vera; WBC, white blood cell. |
All other clinical characteristics, including transfusion dependence, median lactate dehydrogenase levels, and circulating blasts ≥1%, showed no significant difference between the two patient cohorts
Differences in hematologic parameters and a higher frequency of splenomegaly in patients with post-PV MF were consistent with previous reports. The proportion of patients in both groups with constitutional symptoms was also similar. Limitations of the analysis include potential racial bias, due to the study being conducted in Japan, as well as a lack of data on the duration and dosage of ruxolitinib treatment. Overall, this prospective analysis provides valuable insight into real-world patient and prognostic data for these secondary diseases in the ruxolitinib treatment era.
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